scholarly journals Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Patient with Gorlin Syndrome: Spontaneous Partial Resolution of Traction Caused by Epiretinal Membrane

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
José L. Sánchez-Vicente ◽  
Miguel Contreras-Díaz ◽  
Trinidad Rueda ◽  
Enrique Rodríguez de la Rúa-Franch ◽  
Fredy E. Molina-Socola ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Epiretinal Membrane ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Spontaneous Resolution ◽  
Fundus Examination ◽  
Gorlin Syndrome ◽  
Clinical Context

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.

Optical Coherence Tomography Angiography Features of Intrachoroidal Peripapillary Cavitation

2017 ◽  
Vol 27 (2) ◽  
pp. e32-e34 ◽  
Author(s):  
Andrea Mazzaferro ◽  
Adriano Carnevali ◽  
Ilaria Zucchiatti ◽  
Lea Querques ◽  
Francesco Bandello ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Optical Coherence Tomography Angiography ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Fundus Examination ◽  
Optical Coherence ◽  
Inferior Border ◽  
The Right

Purpose To evaluate the optical coherence tomography angiography (OCT-A) features of a peripapillary intrachoroidal cavitation (ICC) in a patient with high myopia. Methods A 67-year-old woman with ICC underwent visual acuity testing, refraction, slit-lamp biomicroscopy, dilated fundus examination, and OCT-A. The main findings are described in this case report. Results Best-corrected visual acuity was 20/20 in both eyes. Fundus examination revealed in the right eye a macular scar, a tilted disc along with a peripapillary staphyloma, and an orange-yellowish lesion on the inferior border of the disc. Structural OCT B-scan showed ICC as an intrachoroidal hyporeflective space located below the normal plane of the retinal pigment epithelium adjacent to the optic nerve head. Optical coherence tomography angiography showed the cavitation as a hyporeflective area, devoid of detectable flow from the choriocapillaris and large choroidal vessels layers, suggesting the choroid, including the residual hyperreflective tissue in the outer aspect of the retinal pigment epithelium/Bruch membrane, to be avascular in ICC. Conclusions Optical coherence tomography angiography demonstrated the absence of choroidal and choriocapillary network.

scholarly journals Long-Term Follow-Up of Congenital Simple Hamartoma of the Retinal Pigment Epithelium: A Case Report

2018 ◽  
Vol 9 (1) ◽  
pp. 215-220 ◽  
Author(s):  
Yuka Ito ◽  
Masahito Ohji
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Japanese Woman ◽  
Initial Examination ◽  
Macular Lesion ◽  
Long Term Follow Up

Background: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign tumor of the retinal pigment epithelium characterized by a focal, darkly pigmented nodule in the macular lesion in healthy persons. We report a case of CSHRPE with long-term follow-up. Case: A 41-year-old Japanese woman was incidentally discovered to have a dark lesion on the fundus of the left eye. We evaluated the patient by measuring her best-corrected visual acuity (BCVA) and by slit-lamp biomicroscopy, fundus color photography, and optical coherence tomography (OCT) over a 10-year period. The BCVA gradually declined during the early follow-up period, having decreased from 1.2 to 0.8 in the left eye 3 years after the initial examination, and then has been maintained for the following 7 years. The lesion did not show a change in OCT 10 years after the first examination. Conclusion: It is important to follow a CSHRPE carefully over the long term because visual acuity might decrease.

Acquired retinoschisis and vitreous hemorrhage as unusual findings in choroideremia: Case report

2020 ◽  
pp. 112067212094657
Author(s):  
Dario Pasquale Mucciolo ◽  
Vittoria Murro ◽  
Dario Giorgio ◽  
Andrea Sodi ◽  
Ilaria Passerini ◽  
...  
Keyword(s):  
Case Report ◽  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Vitreous Hemorrhage ◽  
Advanced Stage ◽  
Vascular Abnormalities ◽  
Diagnostic Difficulties ◽  
The Right

Purpose: To report a case of choroideremia characterized by peripheral retinoschisis with vascular abnormalities and vitreous hemorrhage. Observations: A 58-year-old man affected by advanced-stage choroideremia was diagnosed with peripheral retinoschisis in both eyes. Vitreous hemorrhage was present in the right eye with a peculiar clot-like lesion at the periphery. At the 1-year follow-up, the vitreous hemorrhage had reabsorbed and the vascular clot-like lesion in the periphery had almost completely disappeared. Conclusion and importance: We have reported fundoscopic and OCT features of peripheral-acquired retinoschisis with vascular abnormalities in a patient with choroideremia. OCT examination is extremely useful in clinical evaluation of the peripheral retinal alterations in these cases, where the absence of the retinal pigment epithelium and the choriocapillaris pose many diagnostic difficulties.

Diagnosis and treatment of peripheral exudative haemorrhagic chorioretinopathy

2019 ◽  
Vol 104 (6) ◽  
pp. 874-878 ◽  
Author(s):  
Sarah Vandefonteyne ◽  
Jean-Pierre Caujolle ◽  
Laurence Rosier ◽  
John Conrath ◽  
Gabriel Quentel ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Chart Review ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Choroidal Melanoma ◽  
Endothelial Growth Factor

PurposePeripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options.Material and methodsRetrospective multicentric chart review.ResultsOf 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit.ConclusionIn case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI’s efficacy.

scholarly journals Rapid progression of chorioretinal atrophy in punctate inner choroiditis: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuka Kasuya ◽  
Yuji Inoue ◽  
Satoru Inoda ◽  
Yusuke Arai ◽  
Hidenori Takahashi ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Case Report ◽  
Epiretinal Membrane ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Japanese Woman ◽  
Rapid Progression ◽  
Chorioretinal Atrophy ◽  
Corrected Visual Acuity ◽  
Best Corrected Visual Acuity

Abstract Background The chorioretinal inflammatory lesions occurring in punctate inner choroiditis evolve into punched-out atrophic scars. Typically, the progression is gradual. We report a case of highly myopic punctate inner choroiditis with rapid progression of chorioretinal atrophy. Case presentation A 48-year-old Japanese woman with high myopia presented with decreased visual acuity. Best-corrected visual acuity was 20/28 in the right eye and 20/16 in the left eye; axial length was 29.0 mm and 28.7 mm, respectively. Fundoscopy revealed an epiretinal membrane in the left eye. Three years later, the best-corrected visual acuity in the left eye had decreased to 20/33; at this time, the patient underwent vitrectomy with epiretinal membrane and internal limiting membrane peeling in this eye. Six months later, the best-corrected visual acuity in the left eye decreased suddenly to 20/100. Optical coherence tomography showed a nodule-like lesion in the outer retina with disruption of the retinal pigment epithelium and a focally thickened choroid, compatible with PIC. One month later, the choroidal thickness had decreased. The central chorioretinal atrophy expanded rapidly at a rate of 0.45 mm2/year over the next 3 years, and new areas of patchy focal chorioretinal atrophy developed in the perifovea. Conclusions Rapid progression of chorioretinal atrophy was observed in a patient with punctate inner choroiditis. Because punctate inner choroiditis is often associated with degenerative myopia, the retina is fragile and may be susceptible to mechanical damage. This case report alerts clinicians to the need for careful management of patients with punctate inner choroiditis, especially after vitrectomy.

Multimodal imaging in iodate-induced toxic retinopathy

2020 ◽  
pp. 112067212092686
Author(s):  
Sebile Çomçalı ◽  
Pınar Topcu Yılmaz ◽  
Cemal Çavdarlı ◽  
Mehmet Numan Alp
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Iodine Deficiency ◽  
Vision Loss ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Vitamin Supplementation ◽  
Potassium Iodate ◽  
Intentional Ingestion ◽  
The Right

Introduction Iodine deficiency is a leading cause of preventable physical and mental retardation. Potassium iodate is used for iodine supplementation to prevent iodine deficiency. We herein report a case of toxic retinopathy following intentional ingestion of potassium iodine. Case Presentation A 41-year-old male presented with a 5-day history of blurred vision in both eyes. His visual acuity (VA) was hand motion and his pupillary reactions were sluggish bilaterally. The fundus examination revealed bilaterally diffuse retinal pigment epithelium atrophy and secondary pigmentary changes at the posterior pole, but his peripheral fundus was relatively spared. Choroidal thinning, punctate hyperreflective dots along the retinal pigment epithelium layer, and outer retinal atrophy were the optical coherence tomography findings, which were consistent with widespread areas of retinal pigment epithelium window defects observed on fundus fluorescein angiography. The visual evoked potential test showed no response in the right eye and revealed a delay in the latency and a decrease in the amplitude of the P100 wave in the left eye. Wave b responses of the photoreceptors could not be observed in the patient’s electroretinogram. After a vitamin supplementation protocol consistent with the literature, at the 4-month follow-up visit his visual acuity had improved to 0.3 in the right eye and counting fingers in the left eye. Conclusion Potassium iodate toxicity is a cause of serious retinal and choroidal damage and results in severe vision loss. Hydration, hemodialysis, and antioxidants can be helpful to minimize the complications.

Sjögren Reticular Dystrophy of the Retinal Pigment Epithelium: A Case Report

2003 ◽  
Vol 13 (5) ◽  
pp. 491-495
Author(s):  
M. Rinaldi ◽  
A. Villani ◽  
M. Borrelli ◽  
S. Russo ◽  
L. Cotticelli
Keyword(s):  
Case Report ◽  
Retinal Pigment Epithelium ◽  
Late Onset ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Clinical Manifestations ◽  
Computerized Perimetry ◽  
Fundus Photographs ◽  
First Time

Purpose To describe the clinical manifestations in a patient with Sjögren reticular dystrophy of the retinal pigment epithelium, and the evolution of the disease over a 20-year follow-up period. Case Report A 45-year-old woman with Sjögren reticular dystrophy of the retinal pigment epithelium was seen for the first time in 1983; the patient underwent 20 years of annual check-ups. Results Over the follow-up period, fundus photographs, computerized perimetry, electroretinogram, and electro-oculogram findings had either normal or slightly subnormal outcome. Conclusions The results confirm that this disease involves only the retinal pigment epithelium and should not be considered a central tapeto-retinal degeneration or late onset retinopathy.

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